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MSK > inflammatory vasculitits > Flashcards

inflammatory vasculitits Flashcards

1
Q

what is giant cell arteritis?

A

is the granulomatous inflammation of large cerebral arteries with occurs associated with PMR (poly myalgia rheumatic)

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2
Q

who does giant cell arteritis usually affect?

A

white females >50 yrs

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3
Q

what are 2 types of large vessel vasculitis?

A
  • Takayasu arteritis

- Giant cell arteritis

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4
Q

what are the symptoms of Giant Cell Arteritis (GCA)?

A
  • headache (main one)
  • severe unilateral headache may be noticed when brushing hair
  • jaw claudication
  • blurred or double vision
  • irreversible painless complete sight loss can occur rapidly

systemic symptoms:

  • fever
  • muscle aches
  • weight loss
  • loss of appetite
  • peripheral oedema
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5
Q

how is giant cell arteritis diagnosed?

A
  • clinical presentation
  • raised ESR: usually 50 mm/hour or more
  • temporal artery biopsy
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6
Q

what is found in a temporal artery biopsy for giant cell artiritis?

A

mono nucleated infiltration or granulomatous inflammation usually with multinucleate giant cell

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7
Q

what is the medication for GCA?

A

immediately start 40-60mg of prednisolone- DONT WAIT FOR BIOPSY!

then adjust depending on visual symptoms:
40mg- no visual symptoms
60mg- visual symptoms

aspirin

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8
Q

what is takayasu arteritis?

A

-granulomatous inflammation of the aorta and its major branches

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9
Q

who does takayasu arteritis normally affect?

A

<40
females
asians

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10
Q

what is polymyalgia rheumatica?

A

Chronic inflammatory, systemic condition that affects individuals (>50), associated with finding a giant cell arteritis on temporal artery biopsy

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11
Q

who does polymyalgia rheumatica typically affect?

A

> 50 years

patients with GCA

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12
Q

how does polymyalgia rheumatica present?

A
  • pain and stiffness in the shoulder and hip girdle
  • sudden onset
  • usually symmetrical
  • worse in morning
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13
Q

what are the investigations for polymyalgia rheumatica?

A
  • Raised ESR, plasma viscosity, CRP
  • temporal artery biopsy
  • temporal artery USS
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14
Q

what is the treatment for PMR?

A

-start at prednisolone 15 mg daily

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15
Q

what are the three types of ANCA-associated small cell vasculitis?

A
  • GPA (granulomatous with polyangitis)
  • MPA (microscopic polyangitis)
  • EGPA (eosonophiliic granulomatous with polyangitis)
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16
Q

what was GPA previously called?

A

Wegener’s

17
Q

what was EGPA previously called?

A

Churg-Strauss syndrome

18
Q

what does EGPA often cause?

A
  • often lung problems (asthma)
  • often skin problems
  • less often kidneys
19
Q

what does GPA cause?

A

ENT problems:

  • nose bleeds
  • sinusitis
  • hearing loss

-glomerulonephritis

20
Q

what does MPA cause?

A
  • renal failure

- haemoptysis

21
Q

what is pANCA associated with?

A

MPA and EGPA

22
Q

what is cANCA and PR3 associated with?

A

GPA

23
Q

what investigations are done for ANCA associated small vessel vasculitis?

A
  • immunofloresence to detect the ANCA (eg if it is cANCA or pANCA)
  • inflammatory markers
  • U and Es
  • urinalysis for renal vasculitis
  • CXR
  • biopsy of affected area e.g. skin or kidney
24
Q

what is the treatment for TA (takayasu arteritis)?

A

1st= Steroid sparing agents such as Leflynomide and methotrexate

2nd (if disease doesnt go away after a couple years)= Tocilizumab (IL-6 blocking agent)

25
Q

what is Henoch Shonlein Purapura (HSP)?

A
  • its a type of ANCA negative vasculitis
  • its an acute immunoglobin A (IgA) mediated disorder causing vasculitis of the skin, GI tract, kidneys, joint and reraly the lungs and CNA
26
Q

what immunoglobin mediates Henoch Shonlein Purapura (HSP)?

A

IgA

27
Q

what parts of the body are more commonly affected by Henoch Shonlein Purapura (HSP)?

A

small vessels of the:

  • skin
  • GI tract
  • kidneys
  • joints
  • lungs (rarely)
  • CNS (rarely)
28
Q

what age group does Henoch Shonlein Purapura affect (HSP)?

A

75% of cases occur in children aged 2-11

29
Q

how does henoch shonlein purapura (HSP) present?

A
  • purpuric rash over buttocks and lower leg
  • colicky abdominal pain
  • boody diarrhoea
  • joint pain +/- swelling
  • renal involvement (50%)
30
Q

what is the treatment for ANCA positive small cell vasculitis?

A

Localised (no systemic involvement) = methotrexate + steroids

Generalised/systemic:
1st line= cyclophosphamide + steroids
2nd line= rituximab + steroids

Refractory (unresponsive to other treatment) = IV immunoglobulins and rituximab

31
Q

how long does HSP tend to take to resolve?

A

8 weeks

32
Q

which types of ANCA positive small vessel vasculitis are granulomatous?

A

GPA

EGPA

MSK (64 decks)

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